Capillaroscopic Abnormalities Research Articles

Nailfold capillaroscopic assessment in pediatric patients with autoimmune uveitis: a case-control study.

Uveitis is a major cause of visual impairment. Most uveitis cases have autoimmune etiology. Pediatric autoimmune uveitis may be associated with systemic diseases such as juvenile idiopathic arthritis or may arise as an isolated disorder. It may be accompanied by retinal vasculitis due to retinal microcirculation involvement. Nailfold capillaroscopy, a digital microscope, is a non-invasive tool for systemic microcirculation evaluation. We aimed to evaluate systemic microcirculation abnormalities in pediatric autoimmune uveitis. Twenty-five patients with pediatric autoimmune uveitis and 21 healthy children underwent detailed capillaroscopic evaluation. We assessed capillary density/mm, capillary morphology, capillary dimensions, and the presence or absence of microhemorrhages and avascular areas. The mean age of the study and control groups was 11.24 ± 3.03 and 9.9 ± 4.17years, respectively. Most included patients had isolated uveitis and juvenile idiopathic arthritis (64% and 24%, respectively). The predominant uveitis subtype in the study was anterior uveitis (48%). A significant difference was found between cases and controls regarding mean capillary density (p-value = 0.0003) and the number of subjects having capillary density less than 7 (p-value = 0.002). Other capillaroscopic abnormalities did not show any significant difference between the studied groups. Mean capillary density did not correlate significantly with age, disease duration, or acute phase reactants. Children with autoimmune uveitis, whether isolated or asa part of systemic disease, may have systemic microcirculation involvement. Key Points • Idiopathic autoimmune uveitis is not always an isolated intraocular condition. • Systemic microcirculation involvement may occur in pediatric autoimmune uveitis, even in cases with isolated uveitis. • Nailfold capillaroscopy showed that capillary density in children with autoimmune uveitis is significantly reduced compared to healthy controls.

Nailfold videocapillaroscopy abnormalities and vascular manifestations in Behçet's syndrome.

To evaluate microcirculation abnormalities and their clinical association in patients with BS, especially with vascular manifestations. A cross-sectional study was carried out using nailfold videocapillaroscopy (NVC) to evaluate the microcirculation in patients with BS. A total of 65 patients were included in the study. Thirty-four (52.3%) were men, and 84.6% were European Caucasian. Vascular involvement was present in 24 (36.9%) patients. Qualitative abnormalities in NVC were observed in 47.7% of patients. The most frequent were tortuous and branched capillaries (21.5%), followed by microhaemorrhage (12.3%), enlarged capillary (7.7%) and giant capillary (3.1%). We found a significant relationship between the presence of tortuous and branched capillaries and previous superficial thrombophlebitis (ST) (p=0.025). The presence of ≥2 qualitative abnormalities in NVC was associated with vascular involvement (p=0.031), mainly with venous thrombotic events (p=0.024) and particularly with ST (p=0.003). No specific Cutolo's pattern was observed. No association was observed between NVC and Raynaud's phenomenon or ANA, although patients with positive ANA presented more frequent non-specific capillaroscopic abnormalities (p=0.003). The presence of ≥2 qualitative abnormalities in NVC is associated with Vasculo-Behçet. NVC might be a potential tool for early detection of patients at risk of vascular events.

Relationship between nailfold capillaroscopy findings and the etiology and prognosis of interstitial lung disease

ObjectivesConnective tissue-associated interstitial lung diseases (CTD-ILD) are believed to be caused by microvascular damage. The objective of this study was to assess the nailfold capillaroscopy (NFC) pattern in patients diagnosed with both CTD-ILD and non-CTD-ILD to identify microvascular changes and determine the relation between capillaroscopic parameters, clinical variables, and disease-related measurements.Patients and methodsThis cross-sectional study included 95 patients with interstitial lung disease who applied to our Rheumatology and Chest Clinics between September 2021 and July 2023. The patients were divided into two groups based on their diagnosis: non-CTD-ILD (group 1) and CTD-ILD (group 2). Nailfold capillaroscopy was performed.ResultsNinety-five patients, 49 (51% female, mean age 62.31 ± 11.027 years) in group 1 and 46 (69.6% female, mean age 62.09 ± 10.887 years) in group 2, were included in the study. Abnormal capillary morphologies were both detected in the CTD-ILD group and the non-CTD-ILD groups. In patients with a usual interstitial pneumonia (UIP) pattern on chest computed tomography (CT), tortuosity was higher than in patients with non-specific interstitial pneumonia (NSIP) (P = 0.041), and the proportion of tortuosity increased significantly as the duration of the disease increased (P = 0.016).ConclusionOur study highlights capillaroscopic abnormalities alone may not be sufficient to differentiate CTD-ILD (other than systemic sclerosis) from non-CTD-ILD. The presence of NFC abnormalities in non-CTD-ILD may suggest that fibrotic lung disease could potentially play a role in the deterioration of the microvascular structure or abnormal angiogenesis. Our study demonstrated that a multidisciplinary approach, incorporating clinical, morphological, pathological, and serological evaluations, is necessary for interpreting ILD.Key Points• Capillaroscopic abnormalities can also be seen in non-CTD-ILD.• Capillaroscopy findings do not distinguish the non-Ssc etiology of ILD.• Nailfold capillaroscopy may have the potential to serve as a useful tool in predicting prognosis and monitoring the disease progression in patients with idiopathic pulmonary fibrosis (IPF).

Capillaroscopic abnormalities in systemic lupus erythematosus and its association with clinical manifestation

IntroductionCapillaroscopy is a non-invasive tool used to evaluate microcirculation and determine whether a Raynaud's phenomenon is primary or secondary. Capillaroscopic changes are well-described in systemic sclerosis; however, these alterations have been less studied in other autoimmune diseases. ObjectiveThe aim of this study is to determine videocapillaroscopic alterations in lupus, and its association with clinical manifestations. Materials and methodsA cross-sectional study with analytical intention was performed. Videocapillaroscopy and medical evaluations were performed on 76 patients with lupus, according to SLICC 2012 classificatory criteria, from January to June 2019. Chi2, Fisher, and Mann–Whitney U tests were used to evaluate association, and the prevalence ratios (PR) were determined. A multivariate analysis was performed. ResultsSeventy-one (93.4%) of the patients were female with a median age of 33.5 years (interquartile range [IQR]: 27–44.8); the median lupus duration was 84 months (IQR: 30–168). The main clinical manifestations were articular, cutaneous, hematological, and Raynaud's phenomenon. A non-specific pattern was found in 43 patients (56.6%), and a systemic sclerosis-like pattern was found in 7 patients (9.2%). In bivariate and multivariate analyses, Raynaud episodes occurring more than once a week (PR 1.24; 95% CI: 1.13–1.33) were more frequent in patients with a sclerosis-like or a non-specific pattern. ConclusionLupus patients frequently have videocapillaroscopic alterations with non-specific and systemic sclerosis like patterns, which are more common in patients with Raynaud's phenomenon that occurs more than once a week. Due to the study's design, it is impossible to determine causality.

A Comparison of Nailfold Video Capillaroscopy Findings in Mixed Connective Tissue Disease Interstitial Lung Disease vs Systemic Sclerosis Interstitial Lung Disease: A Single-Centre Study.

To differentiate the nailfold capillaroscopy (NFC) findings in patients with MCTD-ILD and SSc-ILD and correlate the NFC changes and lung functions among them. In this observational study from Oct 2020 to Oct 2022, 27 patients with MCTD-ILD and 27 patients with SSc-ILD were included. NFC was performed using Jiangsu Jiahua, JH 1004, China. Statistical analysis was conducted using IBM SPSS software, version 26, and tests including Mann-Whitney U-test, student t-test, chi-square test, or Fisher's exact test were used to compare between groups. In this study, major capillaroscopic changes were more frequent in SSc-ILD group (92%) than in MCTD-ILD group (72.3%), with normal capillaries seen in 7.4% of MCTD-ILD cases. The mean FVC was higher in SSc-ILD group compared to MCTD-ILD group, and patients with capillary loss had a lower mean FVC. Loss of capillaries was more frequent in SSc-ILD group, while dilated capillaries were predominantly observed in MCTD-ILD group. A significant association was found between the severity of restriction in spirometry and NFC. There is an important role for NFC in detecting the severity of lung involvement, as the grading of restrictive severity in spirometry is strongly associated with capillaroscopic abnormalities.

Non-Invasive Assessment of Micro- and Macrovascular Function after Initiation of JAK Inhibitors in Patients with Rheumatoid Arthritis.

Janus kinase (JAK) inhibitors constitute a novel class of oral biologic disease-modifying antirheumatic drugs for patients with rheumatoid arthritis (RA). However, their use has been associated with increased risk of major cardiovascular events. We investigated whether treatment with JAK inhibitors exerts significant alterations in the micro- and microvasculature in RA patients. Thirteen patients with RA initiating treatment with JAK inhibitors were prospectively studied. Eventually, data from 11 patients who completed the study were analyzed. Procedures were performed at baseline and 3 months after treatment. Nailfold videocapillaroscopy was applied to detect alterations of the dermal capillary network. Participants underwent 24 h ambulatory blood pressure monitoring (Mobil-O-Graph device) for the assessment of blood pressure (both brachial and aortic) and markers of large artery stiffening [pulse wave velocity (PWV), augmentation index] throughout the whole 24 h and the respective day- and nighttime periods. Carotid intima-media thickness was assessed with ultrasound. Three-month treatment with JAK inhibitors was not associated with any differences in brachial and aortic blood pressure, arterial stiffness, and carotid atherosclerosis, with the only exception of nighttime PWV, which was significantly elevated at follow-up. However, three-month treatment with JAK inhibitors induced significant microvascular alterations and increased the total number of capillaroscopic abnormalities. Three-month treatment with JAK inhibitors may exert significant effects on microcirculation as assessed with nailfold videocapillaroscopy, whereas macrovascular structure and function appears largely unaffected. Further research toward this direction may add substantial information to the available literature regarding cardiovascular aspects of JAK inhibitors in RA.

Nailfold capillaroscopy findings of a multicentric multi-ethnic cohort of patients with idiopathic inflammatory myopathies.

To assess nailfold video capillaroscopic (NVC) abnormalities and their association with clinical features, myositis-specific autoantibodies (MSA), and myositis-associated antibodies (MAA) in a large multi-ethnic cohort of patients with idiopathic inflammatory myopathies (IIM). We recruited 155 IIM patients from three centres in Mexico, Spain, and the USA. We evaluated the clinical and laboratory features of the patients and performed semiquantitative and quantitative analyses of the NVC. Each NVC study was defined as having a normal, non-specific, early systemic sclerosis (SSc), active SSc, or late SSc pattern. Twenty-three patients had at least one follow-up NVC when disease control was achieved. Quantitative variables were expressed as medians and interquartile range (IQR) and were compared with the Kruskal-Wallis, the Mann-Whitney U-test, and the Wilcoxon test for paired medians. Associations between qualitative variables were assessed with the χ2 test. Most patients were women (68.3%), Hispanic (73.5%), and had dermatomyositis (DM) (61.2%). Fourteen patients (9%) had a normal NVC. A non-specific abnormality pattern was the most frequent (53.9%), and was associated with joint involvement, interstitial lung disease, Jo1 autoantibodies, anti-synthetase syndrome, and immune-mediated necrotising myopathy. The SSc pattern was observed mostly in DM and overlap myositis and was associated with cutaneous features and anti-TIF-1g autoantibodies. After treatment, there was a decrease in the capillaroscopic score, the capillary diameter, and the number of avascular areas, and an increase in capillary density and bushy capillary number. NVC abnormalities are related to the diagnosis, clinical features, disease activity, and autoantibodies of patients with IIM.

Characterization of patients with idiopathic interstitial pulmonary disease and capillary and laboratory findings in two health institutions in Medellín, Colombia: A descriptive study

IntroductionInterstitial lung disease (ILD) usually has a poor therapeutic response and prognosis. One of the methods that could help in the diagnosis and optimize the management of these patients is capillaroscopy. The study aimed to determine the clinical and capillaroscopic characteristics of patients with ILD and the frequency of findings suggestive of autoimmune disease. Materials and methodsA descriptive observational study that evaluated patients with ILD treated between 2010 and 2019 without a previous diagnosis of autoimmune disease. An interview, capillaroscopy, and laboratory tests were performed. Results28 patients were evaluated, 16 (57.1%) were women and 17 (60.7%) had hypertension. Three patients (10.7%) reported morning stiffness for more than 60 minutes and there was one unexplained digital edema. There were no telangiectasias, Raynaud’s phenomenon, mechanic’s hands, sclerodactyly or Gottron’s sign, or ANAS titres greater than 1:80. The rest of the laboratory tests were negative in 100% of the patients. In the capillaroscopies, 13 (46.4%) patients had a normal capillaroscopic pattern, and 15 (53.6%) had capillaroscopic abnormalities of undetermined significance. There were none with a pattern of systemic sclerosis or similar. ConclusionsNo laboratory or capillaroscopy findings were found that suggested interstitial disease with autoimmune features, possibly due to the low prevalence of the disease, its high mortality, and underdiagnosis. These findings reinforce the concept of capillaroscopic normality in patients with non-autoimmune ILD and call for an active search for ILD with autoimmune features for prognostic purposes.

Capillaroscopic abnormalities in systemic lupus erythematosus and its association with clinical manifestation

IntroductionCapillaroscopy is a non-invasive tool used to evaluate microcirculation and determine whether a Raynaud's phenomenon is primary or secondary. Capillaroscopic changes are well-described in systemic sclerosis; however, these alterations have been less studied in other autoimmune diseases. ObjectiveThe aim of this study is to determine videocapillaroscopic alterations in lupus, and its association with clinical manifestations. Materials and methodsA cross-sectional study with analytical intention was performed. Videocapillaroscopy and medical evaluations were performed on 76 patients with lupus, according to SLICC 2012 classificatory criteria, from January to June 2019. Chi2, Fisher, and Mann–Whitney U tests were used to evaluate association, and the prevalence ratios (PR) were determined. A multivariate analysis was performed. ResultsSeventy-one (93.4%) of the patients were female with a median age of 33.5 years (interquartile range [IQR]: 27–44.8); the median lupus duration was 84 months (IQR: 30–168). The main clinical manifestations were articular, cutaneous, hematological, and Raynaud's phenomenon. A non-specific pattern was found in 43 patients (56.6%), and a systemic sclerosis-like pattern was found in 7 patients (9.2%). In bivariate and multivariate analyses, Raynaud episodes occurring more than once a week (PR 1.24; 95% CI: 1.13–1.33) were more frequent in patients with a sclerosis-like or a non-specific pattern. ConclusionLupus patients frequently have videocapillaroscopic alterations with non-specific and systemic sclerosis like patterns, which are more common in patients with Raynaud's phenomenon that occurs more than once a week. Due to the study's design, it is impossible to determine causality.

Role of short courses on nailfold capillaroscopy in obtaining abilities for the identification of microvasculopathy in patients with Raynaud's phenomenon.

An early distinction between "normal" and "abnormal" capillaroscopic pattern during the first visit to a dermatologist has a crucial significance for a diagnostic management of Raynaud's phenomenon (RP). There exists a question about the level of expertise sufficient to evaluate the microcirculation. To evaluate the utility of short courses on NFC among dermatologists and medical students in obtaining sufficient abilities for the identification of microvasculopathy in patients with RP using videocapillaroscope and handheld dermoscope. Both groups participated in 1-h course on NFC. Before the course, participants were asked to classify 20 videocapillaroscopic and 10 dermoscopic capillaroscopic pictures into "normal" or "abnormal" pattern. Each picture was displayed on a separate slide MS PowerPoint for 10 s. The evaluation was repeated soon after the course. A total of 36 dermatologists and 49 medical students were enrolled. The rate of properly classified dermoscopic and videodermoscopic pictures increased after the course in both groups, but students improved the accuracy of classification on dermoscopic pictures to the greater extent than dermatologists. The rate of correctly recognized pictures with "abnormal" pattern was significantly greater than ones with "normal" pattern at the baseline and after the course, independently of imagining tool. Short courses on NFC may improve the classification of capillaroscopic images, even in medical staff with no previous experience in NFC. The recognition of capillaroscopic abnormalities seems to be easier than obtaining the confidence that evaluated picture has "normal pattern."

Caracterización de pacientes con enfermedad pulmonar intersticial idiopática y hallazgos capilaroscópicos y de laboratorio en dos instituciones de salud en Medellín, Colombia: estudio descriptivo

IntroductionInterstitial lung disease (ILD) usually has a poor therapeutic response and prognosis. One of the methods that could help in the diagnosis and optimize the management of these patients is capillaroscopy. The study aimed to determine the clinical and capillaroscopic characteristics of patients with ILD and the frequency of findings suggestive of autoimmune disease. MethodsA descriptive observational study that evaluated patients with ILD treated between 2010 and 2019 without a previous diagnosis of autoimmune disease. An interview, capillaroscopy, and a laboratory tests were performed. Results28 patients were evaluated, 16 (57.1%) were women and 17 (60.7%) had hypertension. Three patients (10.7%) reported morning stiffness for more than 60minutes and there was one unexplained digital oedema. There were no telangiectasias, Raynaud's phenomenon, mechanic's hands, sclerodactyly or Gottron's sign, or ANAs titres greater than 1:80. The rest of the laboratory tests were negative in 100% of the patients. In the capillaroscopies, 13 (46.4%) patients had a normal capillaroscopic pattern and 15 (53.6%) had capillaroscopic abnormalities of undetermined significance. There were none with a pattern of systemic sclerosis or similar. ConclusionsNo laboratory or capillaroscopy findings were found that suggested interstitial disease with autoimmune features, possibly due to the low prevalence of the disease, its high mortality, and underdiagnosis. These findings reinforce the concept of capillaroscopic normality in patients with non-autoimmune ILD and call for an active search for ILD with autoimmune features for prognostic purposes.

Microvascular capillaroscopic abnormalities and occurrence of antinuclear autoantibodies in patients with sarcoidosis

We described nailfold videocapillaroscopy (NVC) findings and estimated the prevalence of serum anti-nuclear (ANA) and extractable nuclear antigen autoantibodies (ENA) in a cohort of sarcoidosis patients, comparing them with adequate healthy controls (HCs) and with primary Raynaud’s phenomenon patients (PRPs). NVC findings were also correlated with the occurrence of autoantibodies, current treatment, laboratory parameters, variables of lung function and whole-body imaging data. Twenty-six patients with sarcoidosis were assessed through NVC, laboratory parameters, pulmonary function tests, chest-X ray and 18- fluorodeoxyglucose positron emission tomography/computed tomography. The NVC parameters and ANA/ENA dosage were recorded also in 30 PRPs and 30 HCs. Sarcoidosis patients showed a higher rate of capillary dilations and nonspecific abnormalities and a lower mean capillary absolute number than PRPs and HCs (p < 0.01 for all comparisons). The prevalence of ANA positivity was higher in patients with sarcoidosis compared with PRPs and HCs (p < 0.02 for both), whereas ENA positivity was detected in one sarcoidosis patient (Ro52). Among sarcoidosis patients, the mean capillary absolute number negatively correlated with the C-reactive protein concentrations and was positively associated with the forced vital capacity percentage. Instead, a negative correlation was detected between serum ACE levels and the presence of capillary dilations (all p < 0.05). Our findings suggest a microvascular involvement in sarcoidosis whose investigation by NVC might be useful for the follow-up of patients displaying RP. Autoantibody positivity in sarcoidosis might suggest autoimmune implications in the disease or the production of autoantibodies reactive to tissue damage.

Nailfold Microhemorrhage Segmentation with Modified U-Shape Convolutional Neural Network

Nailfold capillaroscopy is a reliable way to detect and analyze microvascular abnormalities. It is safe, simple, noninvasive, and inexpensive. Among all the capillaroscopic abnormalities, nailfold microhemorrhages are closely associated with early vascular damages and might be present in numerous diseases such as glaucoma, diabetes mellitus, and systemic sclerosis. Segmentation of nailfold microhemorrhages provides valuable pathological information that may lead to further investigations. A novel deep learning architecture named DAFM-Net is proposed for the accurate segmentation in this study. The network mainly consists of U-shape backbone, dual attention fusion module, and group normalization layer. The U-shape backbone generates rich hierarchical representations while the dual attention fusion module utilizes the captured features for fine adjustment. Group normalization is introduced as an effective normalization method to effectively improve the convergence ability of our deep neural network. The effectiveness of the proposed model is validated through ablation studies and segmentation experiments; the proposed method DAFM-Net achieves competitive performance for nailfold microhemorrhage segmentation with an IOU score of 78.03% and Dice score of 87.34% compared to the ground truth.

Systemic Sclerosis: Elevated Levels of Leukotrienes in Saliva and Plasma Are Associated with Vascular Manifestations and Nailfold Capillaroscopic Abnormalities.

The role of leukotrienes (LTs) in the pathogenesis of systemic sclerosis (SSc) needs clarification. We analyzed the association of salivary (sa) and plasma (p) levels (pg/mL) of cysteinyl-leukotrienes (CysLT) and LTB4 with SSc vascular manifestations and nailfold capillaroscopy (NFC) in a cross-sectional study. Patients and healthy controls were evaluated for vascular manifestations and NFC. LTs were compared between groups as follows: SSc with or SSc without vascular features and controls, and by NFC parameters. Twenty SSc patients and 16 volunteers were recruited; Raynaud’s phenomenon (RP) history (SSc: saCysLT 99.4 ± 21.8 vs. controls: 23.05 ± 23.7, p = 0.01), RP at examination (SSc: saCysLT 129.3 ± 24.6 vs. controls: 23.05 ± 22.46, p = 0.01; pCysLT SSc: 87.5 ± 11.2 vs. controls: 32.37 ± 10.75, p = 0.002), capillary loss (saCysLT 138.6 ± 26.7 vs. 23.05 ± 21.6, p = 0.0007; saLTB4 3380.9 ± 426.6 vs. 1216.33 ± 346.1, p = 0.0005), “late” scleroderma pattern vs. controls (saCysLT 205.6 ± 32 vs. 23 ± 19.6, p = 0.0002; saLTB4 4564.9 ± 503.6 vs. 1216.3 ± 308.3; p < 0.0001) were all significant. Late patterns had higher levels (saCysLT, p = 0.002; LTB4 p = 0.0006) compared to active and early patterns (LTB4, p = 0.0006), and giant capillaries (p = 0.01) showed higher levels of LTs. Levels of pCysLT were higher in patients with RP at examination vs. patients without RP; saCysLT and LTB4 were higher in SSc group with vs. without capillary loss. LTs could be involved in the pathophysiology of vascular abnormalities. Further research is required to determine if blocking LTs could be a therapeutic target for SSc vascular manifestations.

Nail Fold Capillaroscopy in a Cohort of Egyptian Dermatomyositis/Polymyositis Patients

ABASTRACT Background Nailfold capillaroscopy (NC) has been demonstrated to be an important tool in the diagnosis and treatment of patients with juvenile dermatomyositis (JDM) and systemic sclerosis. However, NC has been scarcely studied in adult patients with DM. Objectives Is to visualize capillary changes in DM/PM patients, trying to find a specific capillary pattern and its correlation if any- with disease activity in those patients. Methodology This is a cross sectional observational study included 20 patients (DM.Diagnosed on base of clinical, laboratory and histopathological findings. We excluded patients with hypertension or diabetes mellitus. The NFC performed using videocapillaroscope. A semiquantitative rating scale was used to score capillaroscopy changes. Myositis disease activity assessment tools were used to assess disease activity [Manual Muscle Testing (MMT), Myositis Disease Activity Assessment Visual Analogue Scales (MYOACT), Health Assessment Questionnaire (HAQ), physician’s VAS, patient’s VAS, skin scoring, serum muscle enzymes levels] Result The median age of our patients was 36 (S.D.12) Mean disease duration was 24months (S.D.19).The results presented mean value 3,8 (S.D.3,3) for muscle disease activity score,2(S.D.3) for skin score and 3,92 (S.D.3,09) for global disease activity .Enlarged capillaries was found in12 patients (100%), avascular areas in 10 patients (83.3%), capillaries hemorrhage in 8 patients (66,7%), and branched capillaries in 6 patients (50%).Sixteen patient (80%) had scleroderma like pattern. We found that disease activity positively correlate with branched capillaries(p = 0,04), muscle disease activity correlate positively with capillaries density score(p = 0,002) while hemorrhage correlate positively with skin score(p = 0,024). In our study major capillaroscopic abnormalities appeared to be virtually confined to patients with DM while patients with PM had only minor changes. No signifcant correlations between NFC and other demographic, clinical and laboratory parameters were observed. Conclusion NFC may provide helpful information about patients with PM/DM by: (a) contributing to the diagnosis and (b) correlating with disease activity parameters. Additional investigations with larger series of patients and prospective studies may be useful to reinforce our data.

Recognition and management of vascular lesions in von Willebrand disease

Recognition and management of vascular lesions in von Willebrand disease

Nailfold Capillaroscopy With USB Digital Microscopy in Connective Tissue Diseases: A Comparative Study of 245 Patients and Healthy Controls.

Background: Nailfold capillaroscopy (NFC) is a valuable tool to detect microcirculation abnormalities in connective tissue diseases (CTDs). However, whether the universal serial bus (USB) digital microscopy used as onychoscopy is as effective as the videocapillaroscopy in determining the diagnostic and prognostic values of CTDs remains to be determined.Objective: This study aims to investigate NFC features of systemic lupus erythematosus (SLE), dermatomyositis (DM), and systemic sclerosis (SSc) patients and compare with normal controls as well as examine which feature could differentiate among CTDs. Furthermore, we aim to explore different capillaroscopic abnormalities and their association with disease activity.Methods: Nailfold images were taken from patients and healthy controls using a USB digital microscopy. Patterns on the capillary morphology, diameter, architecture, and density were recorded and compared. We further determined the NFC findings in SLE, DM, and SSc and corresponded to their respective disease activity scoring system.Results: A total of 245 participants, consisting of 54 SLE, 32 DM, and 51 SSc patients, as well as 108 controls, were enrolled. All capillaroscopic features, except for tortuous capillaries, were significantly more common in CTDs than healthy control (all p < 0.05). A multinomial logistic regression analysis revealed that bushy capillaries had significantly higher odds for both SLE and DM than SSc (OR: 4.10, 95% confidence interval (CI): 1.71–9.81, p = 0.002 and OR: 7.82, 95% CI, 2.86–21.38, p < 0.001, respectively). Elongated capillaries demonstrated significant odds for SLE compared with SSc (OR: 3.35, 95% CI: 1.005–11.20, p = 0.049), while prominent subpapillary plexus showed greater odds for SLE compared with both DM and SSc (OR: 2.75, 95% CI: 1.07–7.02, p = 0.03 and OR: 5.78, 95% CI: 2.29–14.58, p < 0.001, respectively). The presence of hemorrhage, enlarged capillaries, and the low-density index had significantly higher odds in favor of SSc than SLE. Bushy capillaries were the only pattern with a strong association for DM over SSc. The presence of enlarged capillaries indicated higher SLE severity, but no specific finding was related to DM or SSc skin scores.Conclusions: Nailfold capillaroscopic examination using a digital microscope is a valuable method for the diagnosis of SLE, DM, and SSc. Several morphologic patterns can help differentiate among CTDs; however, the prognostic significance of this method requires further investigations.

Comparative characteristics of the clinical and laboratory features of the primary Sjogren’s syndrome associated with anticentromere antibodies and the “classic” subtype of the disease

Objective: to compare clinical and laboratory manifestations in 2 groups of patients with primary Sjogren’s syndrome (pSS): with and without anticentromere antibodies (ACA); compare the incidence, clinical and laboratory characteristics of lymphomas in these two groups.Materials and methods. We examined 119 patients with ACA-positive pSS (pSS-ACA+). pSS was diagnosed based on Russian 2001 criteria, systemic sclerosis (SSc) – criteria ACR/EULAR 2013. To diagnose liver diseases, the level of transaminases, alkaline phosphatase and antimitochondrial antibodies (AMA) was determined, as well as liver biopsy. The diagnosis of primary biliary cholangitis (PBC) was established according to the recommendations of the American Association for the Study of Liver Diseases, the Russian Gastroenterological Association and the Russian Society for the Study of the Liver. Lymphomas were diagnosed according to histological, immunohistochemical and molecular studies of affected organs biopsies, according to the classification of the World Health Organization. A combination of pSS and SSc was diagnosed in 37 patients, and they were excluded from further analysis. We compared clinical and laboratory features in patients with pSS-ACA+ (n=82) and ACA-negative pSS (pSS-ACA–, n=64) and characterized lymphomas in the pSS-ACA+ (n = 14) and pSS-ACA– (n=10) groups.Results and discussion. In patients with pSS-ACA+, a later age of disease onset was revealed, the duration of the disease before lymphoma development did not differ. In patients with pSS-ACA+, we found a lower frequency of rheumatoid factor (RF), antibodies to Ro (anti-Ro) and La (anti-La), decreased C3-complement, hypergammaglobulinemia, increased IgG concentration, CRP, increased ESR, leukopenia and anemia. 51.2% of patients with pSS-ACA+ were seronegative for anti-Ro, anti-La and RF. Patients with pSS-ACA+ had a higher frequency of AMA and elevated IgM. The incidence of cryoglobulinemia and paraproteinemia did not differ. The frequency of recurrent parotitis in pSS-ACA+ was significantly lower, there were no differences in the frequency and severity of other signs of salivary and lacrimal gland damage. PBC and epitheliitis of the biliary ducts in patients with pSS-ACA+ were detected significantly more often. Damage to the peripheral nervous system, lungs, hypergammaglobulinemic purpura, arthralgia and autoimmune thyroiditis were significantly more often detected in the group of patients with pSS-ACA–. In the pSS-ACA+ group, Raynaud’s phenomenon was significantly more frequent, mainly with scleroderma-type capillaroscopic abnormalities. There was no difference in the frequency of other signs characteristic of SSc. MALT lymphomas were diagnosed in the study groups with the same frequency. Patients with lymphomas in the pSS-ACA+ group were characterized by significantly higher laboratory activity. All patients with lymphomas in both groups showed persistent parotid salivary gland enlargement. Lymphomas in both groups developed in patients with late stage salivary and lacrimal gland damage, systemic manifestations of pSS in both groups were rare.Conclusion. pSS-ACA+ is an independent subtype of pSS, which has a number of significant clinical and laboratory differences from the “classic” variant of the disease. ACA in pSS are associated with a low frequency of anti-Ro, anti-La, and RF, as well as an increased risk of PBC and limited SSc. MALT lymphomas in the pSS-ACA+ and pSS-ACA– groups developed with the same frequency and were associated with the progression of glandular damage, regardless of the presence of systemic manifestations.

Systemic Sclerosis

Systemic sclerosis (SSc) is a complex autoimmune rheumatic disease that is characterised by widespread skin and internal organ fibrosis, immune system dysregulation, and vasculopathy. The disease carries a significant burden of pain and disability that is potentially life-limiting because of major internal organ-based complications. Early diagnosis is vital and key investigations include the detection of SSc-associated autoantibodies and nailfold capillaroscopic abnormalities. Patients should be managed by a dedicated, specialist, multidisciplinary team. There is now a range of effective treatments available for many of the complications associated with the disease. Autologous haematopoietic stem cell transplantation may benefit a small subset of patients with very poor prognosis SSc. Important advances have been made in understanding the aetiopathogenesis of SSc, which is driving clinical trials of new therapeutic approaches. The purpose of this review is to provide a clinically focussed description of the relevant aetiopathogenesis, clinical expression of disease, approach to the assessment and treatment of SSc, and highlight the recent advances and future challenges associated with this complex disease.

SAT0601 RELATIONSHIP BETWEEN BODY MASS INDEX AND NAILFOLD CAPILLAROSCOPY IN PRIMARY AND SECONDARY RAYNAUD’S PHENOMENON

Background:Raynaud’s phenomenon (RP) is a diffuse clinical manifestation (3-5% of general population) RP is often secondary to autoimmune systemic diseases, while the condition is classified primary if no underlying disorders can be found. A lower body mass index (BMI) was associated with a greater risk of developing RP, perhaps due to greatest sensivity to cold temperaturesObjectives:The objective of our study was to evaluate the association of BMI with clinical and capillaroscopic features in primary and secondary RP.Methods:Consecutive patients at the first access to a Rheumatology Outpatient Clinic over a 13 months period were screened to RP; nailfold videocapillaroscopy (NVC)was carried out and qualitative and quantitative assessment was performed. Diagnosis of RP was defined in patients who identified color pictures of witnessed attacks.Patients enrolled were screened for secondary causes of RP. RP was classified as primary when no abnormalities were found. Weight and height were collected in clinical records and patients were divided in 3 groups according to their BMI: underweight (BMI&lt;18.5 kg/m2), normal weight (BMI 18.5–25 kg/m2), and overweight (BMI &gt;25 kg/m2). Chi-square test to compare categorical variable and Parametric Student t-test to comparing mean values of normally distributed data were used. p&lt;0.05 was considered to be statistically significant.Results:RP was diagnosticated in 100 of 1416 patients (7.06%). Of these, 73 (10 M, 63F) accepted to undergo NVC. An autoimmune disease was found in 35 patients (47.9%), of which 2 were underweight, 14 normal weight and 19 overweight. Of 38 patients with primary RP,3 were underweight, 23 normal weight and 12 overweight. BMI was significantly higher in secondary RP (p=0.03). Overweight patients with secondary RP were older (p=0.01), but with a disease duration not statistically significant longer (p=0.26). In secondary RP, avascular areas and neoangiogenesis were founded only in overweight patients. Moreover,in secondary RP overweight was correlated with decreased capillary density (p=0-04). There was not association between BMI and capillaroscopic abnormalities in primary RP.Conclusion:In our study BMI was correlated with microvascular changes only in patients with secondary RP. Our findings may suggests a role for obesity in the microcirculatory disfunction in the autoimmune diseases. Further studies are needed to generalize results and to find a causative role.